Pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy.

Abstract

The acute lesions of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) consist of endoneurial foci of chemokine and chemokine receptor expression and T cell and macrophage activation. The myelin protein antigens, P2, P0, and PMP22, each induce experimental autoimmune neuritis in rodent models and might be autoantigens in CIDP. The strongest evidence incriminates P0, to which antibodies have been found in 20% of cases. Failure of regulatory T-cell mechanism is thought to underlie persistent or recurrent disease, differentiating CIDP from the acute inflammatory demyelinating polyradiculoneuropathy form of Guillain-Barré syndrome. Corticosteroids, intravenous immunoglobulin and plasma exchange each provide short term benefit but the possible long-term benefits of immunosuppressive drugs have yet to be confirmed in randomised, controlled trials.

• Authors: Richard A C Hughes, David Allen, Anna Makowska, Norman A Gregson
• Journal: Journal of the peripheral nervous system : JPNS (J Peripher Nerv Syst) Vol. 11 Issue 1 Pg. 30-46 (Mar 2006) ISSN: 1085-9489 [Print] United States
• PMID: 16519780 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)

Chemical References

• Antibodies

Topics

• Animals
• Antibodies (immunology)
• Humans
• Neuritis, Autoimmune, Experimental (immunology, pathology, physiopathology)
• Polyradiculoneuropathy, Chronic Inflammatory Demyelinating (immunology, pathology, physiopathology)
• T-Lymphocytes (immunology)